All Eyes and Ears: A Case of Neurosyphilis Presenting With Simultaneous Ocular and Otic Involvement.

This report details a case of neurosyphilis manifesting as concurrent ocular and otosyphilis, an uncommon presentation of the disease. Here, we describe the diagnosis and treatment of a 27-year-old immunocompetent Caucasian male who presented with uveitis and tinnitus. Physical exam was consistent with uveitis and audiometric testing revealed bilateral sensorineural hearing loss. Serum rapid plasma reagin (RPR) was reactive at 1:512 with a follow-up cerebrospinal fluid (CSF) venereal disease research laboratory (VDRL) test likewise reactive at 1:2, confirming neurosyphilis. The patient was treated with intravenous penicillin G with improvement of symptoms and with subsequent improvement of serum and CSF RPR. However, he ultimately represented with recurrent symptoms and fluctuating serum RPR levels, necessitating repeat treatment and ongoing clinical monitoring. Neurosyphilis can occur at any point during the course of a syphilis infection and may present with a variety of nonspecific findings. This case documents a particularly uncommon instance of simultaneous ocular and otosyphilis, a presentation of neurosyphilis that has only been described a handful of times.

Otosyphilis as a rare cause of secondary benign paroxysmal positional vertigo: a case report.

Otosyphilis is a rare cause of audiovestibular dysfunction that can easily be misdiagnosed. Here, we report a rare case in which a patient presented with secondary benign paroxysmal positional vertigo (BPPV) 2 weeks after symptoms of otosyphilis appeared. The Dix-Hallpike test showed a classical response in the head-hanging left position. The patient was treated with intravenous penicillin G and the canalith repositioning maneuver, which completely resolved the vertigo. The patient's audiovestibular symptoms resolved gradually. The elevated cerebrospinal fluid (CSF) white blood cell (WBC) count returned to normal and the results of the Treponema pallidum particle agglutination (TPPA) test were negative at the 3-month follow-up. This report suggests that otosyphilis should be considered in the differential diagnosis of audiovestibular dysfunction in patients at risk. Additionally, clinicians should remain vigilant about the possibility of secondary BPPV in patients with otosyphilis who report positional vertigo.

Syphilis: A Modern Resurgence.

Syphilis is an important public health problem in the U.S. and many high-income nations. The rates of syphilis continue to increase and there is an urgent need for medical providers of a variety of backgrounds to recognize this disease. In this review, we cover the key clinical findings of syphilis and provide an overview of the diagnosis and management of this disease in adults.

Otosyphilis.

Otosyphilis can be challenging to diagnose, but, if left unrecognized, it may cause irreversible damage. An immunologic interplay between syphilis and human immunodeficiency virus (HIV) makes coinfection likely and may predispose people with HIV to neurosyphilis. In this study, we present a case of a man in his 50s with hearing loss and vertigo diagnosed with otosyphilis as well as a new diagnosis of HIV. This case and corresponding discussion serve to inform the noninfectious disease-trained clinician of the symptoms, diagnostics, and treatment options for otosyphilis as well as to discuss the relationship between HIV and syphilis and demonstrate the importance of disease recognition.

Otosyphilis: A rare case of reversible hearing loss in a young man with secondary syphilis.

Background: Over the last decade, it has been noticed a significant increase in the number of cases of syphilis with a concurrent increased number of patients presenting with syphilis-related complications. Otosyphilis is a well-known complication of syphilis that most of the time, can lead to irreversible hearing loss, especially with delayed diagnosis and treatment. A high index of suspicion is needed for an accurate diagnosis of otosyphilis. Complete audiologic recovery is rare but still possible with the appropriate treatment. Case report: Herein, we describe a case of reversible hearing loss secondary to otosyphilis in a young healthy man who was initially diagnosed and treated as a case of secondary syphilis, and presented later to the clinic with unilateral tinnitus and hearing loss. Audiology findings were consistent with asymmetric sensorineural hearing loss. Fortunately, complete recovery of hearing was achieved after treatment with a 14-day course of intravenous penicillin. Conclusion: Otosyphilis is one of the rare presentations of syphilis; thus, the diagnosis is often missed or delayed. Prompt diagnosis and treatment can help prevent the occurrence of permanent hearing loss.

An Updated Review of Recent Advances in Neurosyphilis.

Neurosyphilis is caused by Treponema pallidum invading the central nervous system, of which the incidence is increasing worldwide. Due to its variable clinical manifestations, diagnosis of neurosyphilis remains challenging, especially the asymptomatic form. This review focuses on recent advances in neurosyphilis, including epidemiology, clinical manifestations, laboratory findings, comorbidities, diagnosis, treatment, prognosis, and basic research. The expansion of men who have sex with men and the infection of human immunodeficiency virus mainly accounted for the increasing incidence of neurosyphilis. The rate of some historically described forms of neurosyphilis in the pre-antibiotic era declined significantly; atypical features are more prevalent. Neurosyphilis, regarded as a great mimicker for neuro-ophthalmic, audio-vestibular, and psychiatric disorders, often presents concomitantly with other diseases, including metabolic disorders. Studies on long non-coding RNAs, miRNAs, chemokines, and metabolites in peripheral blood and cerebrospinal fluid may facilitate exploring the pathogenesis and identifying novel biomarkers of neurosyphilis. The drug resistance of Treponema pallidum to penicillin has not been reported; ceftriaxone was proposed to be more effective than penicillin, whereas few randomized controlled trials supported this view. This study may pave the way for further research, especially the diagnosis and treatment of neurosyphilis.

Magnetic Resonance Imaging in Otosyphilis: A Rare Manifestation of Neurosyphilis.

We report magnetic resonance imaging (MRI) in a case with otosyphilis, which is a rare manifestation of neurosyphilis. A 50-year-old male presented with progressive hearing loss on the left side and recent-onset palsy of the left 7th and 12th cranial nerves. Computed tomography imaging showed destructive bone lesions involving the petrous temporal bone, middle ear, and mastoid region with a pathognomonic pattern of bone destruction depicted in the volume rendering technique images. MRI showed features of destructive bone lesions (gummatous lesion), meningoneuritis, and labyrinthitis. Pathological examination and treponemal antibody absorption test favored the diagnosis. Otosyphilis should be considered as a differential diagnosis in an adult patient with destructive lesions of the petrous temporal bone. To the best of our knowledge, MRI in otosyphilis has not been reported.

Neurosífilis con neuropatía óptica y auditiva simultáneas en un paciente VIH positivo / Neurosyphilis with simultaneous optic and auditive involvement in an HIV-positive patient

Introducción: a partir del año 2000, los médicos han asistido a un retorno de la sífilis vinculado a prácticas sexuales no protegidas y con parejas múltiples, en especial entre hombres que tienen sexo con hombres. La coinfección Treponema pallidum/virus de la inmunodeficiencia humana (VIH) modifica tanto la historia natural de la sífilis, incrementando la incidencia de neurosífilis temprana, como la respuesta al tratamiento con penicilina. Métodos: un paciente varón, peruano, de 36 años, seropositivo para VIH, consulta por dis-minución de la agudeza visual en ojo derecho, pérdida de la audición, tinnitus, mareos y vértigo. Refería antecedentes de sífilis en los 2 años previos. Resultados: el examen oftalmológico efectuado al paciente mostró células en el segmento anterior del ojo derecho. El fondo de ojo reveló la existencia de inflamación del nervio óptico asociada con panuveítis. En base a los hallazgos clínicos, los valores de VDRL en suero y líquido cefalorraquídeo (LCR) se diagnosticó neurosífilis (NS) con neuritis óptica, panuveítis y otosífilis en un paciente coinfectado por VIH. El paciente fue tratado con penicilina G sódica intravenosa, 4 millones de UI cada 4 horas, durante 2 semanas con buena respuesta clínica. Discusión: el compromiso de los pares craneales óptico y auditivo puede representar la manifestación de una NS temprana, en especial, en el contexto de un paciente VIH positivo. De acuerdo con nuestro conocimiento, este sería el segundo caso publicado de compromiso simultáneo del nervio óptico y del aparato vestíbulo-coclear en un paciente con NS.

Introduction: from the 2000s, the physicians experienced a return of syphilis, which may be related to unrestricted sexual behaviour with unprotected contact between multiple partners, especially in men who have sex with men. Concurrent infection with human immunodeficiency virus (HIV) alters the natural history of syphilis by increasing the frequency of early neurosyphilis and the response to penicillin. Methods: a 36-year-old Peruvian man, seropositive for HIV infection, was admitted to the hospital with decreased visual acuity in his right eye, hearing loss, tinnitus, buzzing, and vertigo. He referred history of syphilis in the previous two years. Results: oph-thalmological examination was performed. Ocular anterior segment examination of the right eye showed cells. Fundoscopy revealed swelling of the right optic disc with panuveitis. Diagnosis of neurosyphilis (NS) with optic and ear neuritis, and concurrent HIV infection was made, based on the clinical manifestations and serum and cerebrospinal VDRL titers. The patient was treated with intravenous penicillin (four million units every four hours for two weeks) with an excellent clinical response. Discussion: simultaneous optic and auditive cranial nerve involvement can manifest early neurosyphilis (NS) and HIV coinfection. This is the second report to describe the simultaneous occurrence of syphilitic optic neuritis with vestibulocochlear nerve involvement.

Otosyphilis: A Rare Cause of Reversible Hearing Loss in a Teenage Male.

A high index of suspicion and a thorough neurotologic examination at the onset of presentation are imperative to generate the diagnosis of otosyphilis. Complete audiologic recovery is rare but possible in approximately 20%-25% of patients after appropriate treatment. We present a case of reversible hearing loss secondary to otosyphilis in a teenage male patient with a new diagnosis of human immunodeficiency virus (HIV). Audiology findings were consistent with mixed hearing loss. Lumbar puncture results were consistent with neurosyphilis. Prompt treatment with a 14-day course of intravenous penicillin led to the complete recovery of hearing. In this case report, the pathophysiology, symptomology, and management of otosyphilis are discussed.

Otosyphilis: A rare cause of acute bilateral sensorineural hearing loss in a HIV-negative patient.

Bilateral acute hearing loss is rare, and the aetiology is poorly defined. Less common treatable pathologies such as otosyphilis must be part of the differential diagnosis and should be actively excluded. We present a case of a 23-year-old woman who developed acute bilateral hearing loss due to otosyphilis, confirmed on audiometry and laboratory tests. In this article, the CT, MRI and clinical findings are presented and discussed.

Intrathecal Synthesis Index of Specific Anti-Treponema IgG: a New Tool for the Diagnosis of Neurosyphilis.

Neurosyphilis (NS) diagnosis is challenging because clinical signs are diverse and unspecific, and a sensitive and specific laboratory test is lacking. We tested the performance of an antibody index (AI) for intrathecal synthesis of specific anti-Treponema IgG by enzyme-linked immunosorbent assay (ELISA) for NS diagnosis. We conducted a retroprospective monocentric study including adults with neurological symptoms who had serum and cerebral spinal fluid (CSF) samples collected between 2006 and 2021. Two NS definitions were used. NS1 included patients with neurological symptoms, positive Treponema pallidum particle agglutination (TPPA) serology, and CSF-TPPA of ≥320, as well as CSF-leukocytes of >5 cells/mm3 and/or CSF-protein of >0.45 g/L and/or a reactive CSF-VDRL/RPR test. NS2 included patients with acute ocular and/or otologic symptoms, positive TPPA serology, and a response to NS treatment. Controls were patients with central nervous system disorders other than neurosyphilis. Anti-Treponema pallidum IgG were measured simultaneously in serum and CSF, and AI was calculated according to Reiber diagram. We assessed the AI test area under the curve (AUC), sensitivity/specificity, and estimated positive and negative predictive values. In total, 16 NS1 patients, 11 NS2 patients, and 71 controls were included. With an AI of ≥1.7 as a positive test for NS diagnostic, specificity was 98.6% (95% confidence interval [CI 95%] of 92.4 to 100.0) and sensitivity was 81.3% (CI 95% of 54.4 to 96.0) for NS1 and 98.6% (CI 95% 92.4 to 100.0) and 27.3% (CI 95% 6.0 to 61.0), respectively, for NS2. Positive and negative predictive values were >95% for NS1 and >85% for NS2, for prevalence above and below 20%. Measuring an AI for intrathecal synthesis of specific anti-Treponema pallidum IgG is a new promising tool highly specific for NS diagnosis. IMPORTANCE In the context of a lack of a gold standard for the diagnosis of neurosyphilis due to either nonspecific or nonsensitive tests, we present in this article a new promising tool highly specific for NS diagnosis. This new test involves measuring an intrathecal synthesis index of specific anti-Treponema IgG by ELISA.

Acute Nephrotic Syndrome and Otosyphilis.

Syphilis, also known as the "the great masquerader," is a re-emerging infectious disease in the Western world, and may present with various signs and symptoms, making it difficult to distinguish from other diseases. Nephrotic syndrome due to syphilis has been rarely reported in modern times. Here, we describe a young male with acute hearing loss and peripheral edema, found to have acute nephrotic syndrome in the setting of otosyphilis. Given increasing incidence of syphilis, physicians must remain alert to syphilis as a possible cause of nephrotic syndrome.

A Case of Neurosyphilis and Review of Manifestations, Diagnosis, and Treatment.

Diagnosis of neurosyphilis remains a challenge due to no existing standardized testing, but it is often made based on a combination of clinical and cerebrospinal fluid (CSF) analysis findings. Neurosyphilis is uncommon now compared to the era before the introduction of penicillin. Syphilis if left untreated may lead to debilitating complications including paresis, progressive dementia, and even death. Presence of ocular or hearing manifestations with positive serum treponemal and non-treponemal tests are diagnostic for neurosyphilis, regardless of presence or absence of CSF abnormalities. The preferred regimen for neurosyphilis is intravenous penicillin G for 2 weeks. Other regimens are not shown to be as effective as penicillin. Here we discuss an interesting case presenting with neurosyphilis along with manifestations consistent with primary and secondary stages of syphilis.

Hearing loss in individuals at risk for neurosyphilis.

Otosyphilis is a serious complication of syphilis.329 participants enrolled in a study of cerebrospinal fluid (CSF) abnormalities in syphilis underwent portable audiometry (250 Hz to 8000 Hz at 5-75 dB); it was repeated in 33 after otosyphilis treatment. Treponema pallidum spp pallidum (T. pallidum) DNA in blood was quantitated by polymerase chain reaction. Odds ratios (ORs) or hazard ratios (HRs) with 95% confidence intervals (CIs) were determined by logistic, ordinal or Cox regression.166 (50.5%) had normal hearing; 15 (4.6%) had low frequency (LF) loss alone, 93 (28.3%) had high frequency (HF) loss alone, and 55 (16.7%) had both. Adjusted odds of any hearing loss were higher with detectable blood T. pallidum DNA (3.00 [1.58-5.69], p = 0.001), CSF pleocytosis (2.02 [1.12-3.66], p = 0.02), and older age (2.22 per 10-year increase, [1.70-2.91], p < 0.001). HRs of normalization of LF and HF loss were lower for older individuals (0.20 [0.07-0.63, p = 0.005] and 0.22 [0.05-0.94, p = 0.04]), and HRs for normalization of HF loss were lower for those with more severe loss (0.09 [0.02-0.43], p = 0.002), and in those with CSF pleocytosis (0.32 [0.11-0.96], p = 0.04).Older age and CSF pleocytosis increase the likelihood of otosyphilis and impair hearing recovery after otosyphilis treatment.

Pediatric otosyphilis-An unusual cause of conductive hearing loss.

We present the case of a 5 year old female with a unilateral conductive hearing loss which had a relapsing and remitting course over a 3 year period. An initial noncontrast CT temporal bone study was unremarkable and a diagnosis of otitis media was made in the first instance. However, a second CT temporal bone study performed 3 years later demonstrated bilateral demineralisation of the ossicles and abnormal lucency affecting both the otic capsules. A diagnosis of otosyphilis was proposed on the basis of the imaging features and a prior medical history of previously treated congenital syphilis. With the benefit of hindsight, early pericochlear lucency was identified on the initial CT temporal bone study. There has been a steady rise of syphilis cases since the millennium with resurgence in many high income countries. Otosyphilis has a highly variable clinical presentation and there is limited data to establish the pattern of hearing loss in pediatric patients with a background of congenital otosyphilis. Temporal bone and otic capsule demineralisation carries a broad differential diagnosis including osteogenesis imperfecta, otosclerosis, Paget's disease and radiation related changes. Otosyphilis is a rare but potentially treatable cause of deafness and a high index of suspicion is required to make the diagnosis. In conjunction with a positive syphilis serology, a noncontrast temporal bone CT can aid the diagnosis and expedite the treatment.

Penicillin G Sodium as a Treatment of Otosyphilis with Hearing Loss.

Otosyphilis is one contributing cause of hearing loss in adult patients. There are limited studies on the treatment regimens of otosyphilis. Penicillin G sodium (PGS) plus additional medications, such as benzathine penicillin and probenecid, is an effective regimen. This study investigated the efficacy of PGS alone for the treatment of otosyphilis. We conducted a retrospective study and included all consecutive patients diagnosed with otosyphilis who received only PGS treatment. The study period was from 2009 to 2013. The PGS treatment regimen was PGS 4 mu intravenously every four hours (24 mu/day) for 14 days. Clinical and audiogram outcomes were evaluated one year after treatment. There were 34 otosyphilis patients that were treated with PGS. After one year of treatment, 18 patients (52.9%) had a clinical improvement and 11 patients (32.4%) had an audiogram improvement. In conclusion, PGS at 24 mu/day for two weeks provided an audiogram improvement one year after treatment in one-third of the patients.

Otosyphilis: Resurgence of an Old Disease.

OBJECTIVES: To describe the clinical characteristics of patients presenting with a new diagnosis of otosyphilis over the past 10 years in a large, urban, safety-net hospital affiliated with a large county sexually transmitted disease clinic. METHODS: Retrospective case series. A chart review was performed of all patients who presented to an adult otolaryngology clinic with a new diagnosis of syphilis and hearing loss from January 2008 to December 2017. RESULTS: Twelve patients met the criteria for "suspected" or "likely" otosyphilis based on Centers for Disease Control and Prevention definitions. The average age was 48 years (range 19-59). All were male. Nine (75%) were men who have sex with men. Eight (67%) were positive for human immunodeficiency virus. One (8%) presented with primary, nine (75%) with secondary, and two (17%) with early latent syphilis. Seven (58%) presented with bilateral audiogram-confirmed hearing loss, two (17%) with unilateral hearing loss, and three (25%) with suspected hearing loss based on fluctuating symptoms. Nine (75%) presented with tinnitus and two (17%) with vertigo. The median duration of otologic symptoms prior to presentation was 2 weeks (range: 0-16 weeks). All presented within the last 2 years surveyed. CONCLUSION: We have seen an increase in the number of otosyphilis cases in our clinic. We suspect otosyphilis may be underdiagnosed and emphasize the importance of screening for syphilis in patients with new audiologic symptoms of vertigo, tinnitus, or hearing loss. LEVEL OF EVIDENCE: 4 Laryngoscope, 129:1680-1684, 2019.

[Vertigo and sensorineural hearing loss due to syphilis: differential diagnosis and treatment].

Objective:To analyze the etiology of repeatedly attacks of intractable vertigo and some types of sensorineural deafness whose clinical manifestation were not in conformity with the known spectrum diseases，and explore the screening method to prevent missed diagnosis or misdiagnosis, then provide references for clinical diagnosis and treatment for rare etiology. Method:The authors retrospectively analyzed the clinical manifestations, diagnosis, treatment and prognosis from 4 cases of vertigo sufferers and 2 cases of hearing impairment sufferers whose serological tests were positive for syphilis. All these 6 cases were treated with large doses of penicillin aqueous solutions (24 million U/d), multi-times intravenous infusion, the course of the treatment was 14 d. Result:The clinical manifestations of these 6 patients were lack of characteristic, as well as the results of hearing and vestibular function, imaging diagnosis. Positive syphilis detection of serology and cerebrospinal fluid tests were the main diagnostic basis. After anti-syphilis treatment, 5 cases got satisfied clinical symptoms improvement, 1 case suffered from low-tone sensorineural hearing loss, whose hearing fluctuated recurrently. Conclusion:Syphilis infection may damage the Ⅷ cranial nerve and then lead to vertigo and hearing loss, through chronic syphilitic osteitis of temporal bone, atrophy of organ of corti, osteolytic lesion surrounding the endolymphatic duct, and neurosyphilis. For patients presented with intractable vertigo, and those whose clinical manifestations are not in conformity with the known diseases of unilateral ear or bilateral ears rapidly progressive deafness, syphilis serology screening and validation tests are recommended in case of missed diagnosis or misdiagnosis.

Vertigo and sensorineural hearing loss due to syphilis: differential diagnosis and treatment / 临床耳鼻咽喉头颈外科杂志

Objective:To analyze the etiology of repeatedly attacks of intractable vertigo and some types of sensorineural deafness whose clinical manifestation were not in conformity with the known spectrum diseases，and explore the screening method to prevent missed diagnosis or misdiagnosis, then provide references for clinical diagnosis and treatment for rare etiology. Method:The authors retrospectively analyzed the clinical manifestations, diagnosis, treatment and prognosis from 4 cases of vertigo sufferers and 2 cases of hearing impairment sufferers whose serological tests were positive for syphilis. All these 6 cases were treated with large doses of penicillin aqueous solutions (24 million U/d), multi-times intravenous infusion, the course of the treatment was 14 d. Result:The clinical manifestations of these 6 patients were lack of characteristic, as well as the results of hearing and vestibular function, imaging diagnosis. Positive syphilis detection of serology and cerebrospinal fluid tests were the main diagnostic basis. After anti-syphilis treatment, 5 cases got satisfied clinical symptoms improvement, 1 case suffered from low-tone sensorineural hearing loss, whose hearing fluctuated recurrently. Conclusion:Syphilis infection may damage the Ⅷ cranial nerve and then lead to vertigo and hearing loss, through chronic syphilitic osteitis of temporal bone, atrophy of organ of corti, osteolytic lesion surrounding the endolymphatic duct, and neurosyphilis. For patients presented with intractable vertigo, and those whose clinical manifestations are not in conformity with the known diseases of unilateral ear or bilateral ears rapidly progressive deafness, syphilis serology screening and validation tests are recommended in case of missed diagnosis or misdiagnosis.